POS0760 HIGH LEVELS OF PROTEINS, CELL COUNTS, AND IL-6 IN CEREBROSPINAL FLUID IN PATIENTS WITH NEUROPSYCHIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS ARE ASSOCIATED WITH DAMAGE ACCRUAL OF NON-NEUROPSYCHIATRIC DOMAINS AS WELL AS THAT OF NEUROPSYCHIATRIC DOMAINS
Authors: M. Edamoto, J. Kikuchi, H. Hanaoka, S. Saito, K. Hiramoto, Y. Kaneko
Journal: Annals of the Rheumatic Diseases (2022)

Background Neuropsychiatric systemic lupus erythematosus (NPSLE) is one of severe organ manifestations and directly associated with neuropsychiatric damages, such as cerebral vascular accident, cognitive impairment, or seizures. However, factors associated with damage accrual of neuropsychiatric and non-neuropsychiatric domains in NPSLE patients remain unknown. Objectives The aim of this study was to identify factors associated with damage accrual in patients with NPSLE. Methods We reviewed all patients with SLE who had attended our hospital between January 2010 to December 2020 retrospectively and identified those with NPSLE. We analyzed clinical characteristics and laboratory data including cerebrospinal fluid (CSF) in association with the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) damage index (SDI) progression after the onset of NPSLE. Results 461 patients with SLE were reviewed. Among them, 37 (8.0%) were diagnosed with NPSLE. Thirty-six patients were included in the analysis after excluding one patient with insufficient information. Seventeen (47.2%) patients were diagnosed with NPSLE at the same time with SLE diagnosis and 19 patients were diagnosed at relapse. At NPSLE onset, the mean age was 33.7 years, 35 were female, and the mean SLEDAI-2K was 21.8. Eighteen (50.0%) patients had active lupus nephritis simultaneously at the onset of NPSLE. The most frequent NPSLE types according to the 1999 ACR classification were lupus headache in 10 patients, followed by cerebrovascular disease in 7, seizure disorders in 6, acute confusional state, psychosis and mononeuropathy in 3, and aseptic meningitis in 2. All patients were treated with glucocorticoids, and the mean initial dose of prednisolone was 52.3 mg/day with steroid pulse therapy in 19 patients. Thirty (83.3%) patients were treated with concomitant immunosuppressive treatments. During the observation period with the mean of 10.0 years, 17 (47.2%) patients developed at least one point of SDI. Among them, 9 patients (25.0%) had neuropsychiatric damage progression including cerebrovascular accident in 6, seizures requiring drug treatment for more than 6 months in 2, and cognitive impairment in 1. Non-neuropsychiatric damage was also increased such musculoskeletal in 5, ocular in 3, cardiovascular in 2, renal, gastrointestinal, premature gonadal failure, diabetes, and malignancy in each one case. Neither neuropsychiatric nor overall damage progression were associated with SLEDAI-2K, anti-cardiolipin antibodies positivity, abnormal electroencephalogram, abnormal single photon-emission computed tomography at NPSLE onset or kinds of immunosuppressive treatments. In 21 patients who underwent CSF examination, the high level of CSF IL-6 was associated with neuropsychiatric damage progression (p=0.032). Also, high levels of CSF protein (p=0.030), cell counts (p=0.007), and IL-6 (p=0.032) were associated with overall SDI progression. Conclusion CSF IL-6 concentrations are associated with neuropsychiatric damage progression, and high CSF protein, cell counts and IL-6 at onset in patients with NPSLE can predict overall damage accrual. References [1]Liang MH, Corzillius M, Bae SC, et al. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999; 42: 599–608. [2]Gladman D, Ginzler E, Goldsmith C, et al. The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for systemic lupus erythematosus. Arthritis Rheum 1996; 39: 363–369. Disclosure of Interests None declared